Open heart surgery with mitral valve replacement - ordeal of an undiagnosed haemophilia patient.

Ghosh, Kanjaksha; Madkaikar, Manisha; Jijina, Farah; Gandhi, Sweeta D.; Shetty, Shrimati; Mohanty, Dipika

doi:10.1046/j.1365-2257.2003.00498.x

Cited by 10 publications

(14 citation statements)

References 13 publications

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“…Ghosh et al [17] described a 27-year-old male with unrevealed haemophilia A who underwent MVR (mechanical) without any correcting factor replacement therapy. He developed severe postoperative bleeding.…”

Section: Resultsmentioning

confidence: 99%

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Rossi

Jayaram

Sayeed

2011

Interactive CardioVascular and Thoracic Surgery

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A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether patients with haemophilia undergoing cardiac surgery have good surgical outcomes. Haemophilia A and haemophilia B are sex-linked recessive inherited diseases affecting males only, with females acting as carriers. The conditions result in various degrees of factor VIII or factor IX deficiency, respectively. The life expectancy of haemophilia patients is increasing and now approaches that of the general male population, and they are confronted with age-related co-morbidity, including ischaemic cardiovascular disease. Replacement of the deficient factor (VIII for haemophilia A and IX for haemophilia B) is the cornerstone of treatment; other therapeutic options include tranexamic acid, desmopressin and aprotinin. Recently, the advent of recombinant factor VIII and IX has eliminated the infective risk of using factor concentrates, such as prothrombin complex concentrate or fresh frozen plasma. A total of 84 papers were found using the reported search criteria, and out of this 25 papers, selected with reference to a more modern date range, provided the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results were tabulated. We conclude that there is lack of good-quality evidence and that, in all probability, these papers are subject to publication bias as poor outcomes are unlikely to have been reported. However, all the reported series showed that good outcomes are possible in this specific subgroup of patients given the correct approach. The data accrued from these studies (a total of 30 adults and three children) suggest that routine cardiac surgery can be performed safely in patients with haemophilia, with minimal morbidity and mortality. We identified the following key points to achieve this result: a team approach, a factor replacement protocol and perioperative monitoring of factor levels. Intraoperative plasma factor levels can be easily measured before heparin and after protamine sulphate administration, whereas during cardiopulmonary bypass this will require a chromogenic method. Exposure to factor concentrates early in the life might predispose a patient with severe haemophilia to the development of inhibitors. Moreover, the absence of inhibitors should be confirmed before any surgical procedure.

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Section: Resultsmentioning

confidence: 99%

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Rossi

Jayaram

Sayeed

2011

Interactive CardioVascular and Thoracic Surgery

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“…Generally anticoagulation in haemophilia patients in western countries has been given along with factor replacement therapy under various circumstances. Although this is rational for a developing country like ours it may not be always feasible for financial reasons to give these patients regular factor replacement [8,9].…”

Section: Peri-operative Managementmentioning

confidence: 96%

Successful Aortic Valve Replacement Surgery in a Patient with Severe Haemophilia a with Low Titre Inhibitor

Damodar

Bhat

Kumar

et al. 2013

Indian J Hematol Blood Transfus

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In spite of the modern day innovations, managing severe Haemophilia patients with inhibitors continues to be a challenge. The management of patients with severe haemophilia with inhibitors who are undergoing major surgeries like open heart surgery is technically demanding, fraught with peri-operative complications and needs a multidisciplinary approach. We describe a young man with severe haemophilia with low titre inhibitors who underwent a successful open heart surgery and aortic valve replacement, supported only with bolus doses of Factor VIII and tranexamic acid without any complications.

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“…In haemophiliacs where genitourinary bleeding is persistent or recurrent, a thorough investigation of the genitourinary tract is indicated. In 19 such patients investigated, six were found to have urinary tract stones (Ghosh et al. , 2003a,b; Ghosh, Jijina & Mohanty, 2003c).…”

Section: Acute Bleedingmentioning

confidence: 99%

“…Most joint bleeds are treated without factor concentrates (Dietrich, 1996). A significant proportion of patients (10–20%) develop disabling chronic synovitis (Ghosh et al. , 2003a,b; Ghosh, Jijina & Mohanty, 2003c).…”

Section: Consequences Of Repeated Bleedingmentioning

confidence: 99%

Management of haemophilia and its complications in developing countries

Ghosh

2004

Clin Lab Haematol

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Eighty per cent of people with haemophilia live in developing countries, where technical expertise and health care facilities may be less than optimal. Haemophilia is a relatively rare disease and high-cost, technology-intensive therapy is not a high priority for the governments of developing countries. The rapid spread of transfusion-related viral infections in many developing countries presents further problems for haemophiliacs. However, it is possible to manage haemophiliacs patients with limited resources. Strategies for conserving factor concentrates, include education of doctors and patients, prenatal diagnosis, increasing the use of anti-fibrinolytic agents, physiotherapy, the use of fibrin glue, and simple orthotics and prosthetic measures. These approaches are helpful in the majority of these patients. Meanwhile, with the help of the World Federation of Haemophilia (WFH), all developing countries are gradually improving management skills for this relatively rare but disabling disease. The present review broadly describes the management of various aspects of severe haemophilia in developing countries.

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Open heart surgery with mitral valve replacement - ordeal of an undiagnosed haemophilia patient.

Cited by 10 publications

References 13 publications

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Successful Aortic Valve Replacement Surgery in a Patient with Severe Haemophilia a with Low Titre Inhibitor

Management of haemophilia and its complications in developing countries

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